Pediatric germ cell tumor(GCT) with extensive tumor thrombus of inferior vena cava(IVC) presenting as an oncological emergency is extremely rare .Thirteen months female child with sacrococcygeal GCT having IVC and right atrial thrombus presenting with anasarca and respiratory distress is reported. Due to worsening symptoms post initiation of chemotherapy tumour thrombus debulking from right atrium and distal end of IVC was performed on day 9 on emergency basis. Her symptoms improved and after planned chemotherapy tumor excision with coccygectomy could be done. The IVC thrombus remained inoperable due to sclerosis. She remains recurrent-free now 12 months post therapy.

Xeroderma pigmentosum (XP) is an autosomal recessive disorder caused by defect in nucleotide excision repair and is associated with increased incidence of skin cancer. Lymphoblastic leukemia in XP is rare. We report a case of a 11-year-old boy with XP, who developed B – Acute lymphoblastic leukaemia (ALL). He was started on Modified BFM (Berlin-Frankfurt-Münster) ALL Protocol. Tolerance to ALL chemotherapy was good in our XP patient except for the transient derangement of liver function tests during induction, Pseudomonas sepsis and oral mucositis during protocol M and hyperbilirubinemia during maintenance chemotherapy. Though rare, children with XP requires to be investigated for acute leukaemia on development of bicytopenia.